Prematurity and Congenial Diaphragmatic Hernia: Revisiting Outcomes in a Contemporary Cohort

Objective

To describe outcomes in a contemporary cohort of infants born at gestational age <34 weeks with congenital diaphragmatic hernia (CDH).

Study design

Data from the multicenter, multinational CDH Study Group (CDHSG) registry on patients born between 1995 and 2022 were analyzed. Variables including prenatal characteristics and therapy, postnatal management, and outcomes to discharge were evaluated. A t test was used for continuous variables, and for categorical variables, the Fisher exact test.

Results

In total, 13 471 patients with CDH were entered into the registry during the study period. 810 (6%) were born at < 34 weeks of gestational age. The survival rate for the preterm cohort was 45%, and 43.5% were not repaired. Survival after surgical repair was 79%, mean age at surgery was 11 days of life, and 65% required patch repair. Extracorporeal life support was used in only 7% of the cases, and 35% survived. In total, 48.9% had an associated anomaly. Oxygen at 30 days of life was required by 76.7%. Very preterm birth (<32 weeks of gestational age) occurred in 361 patients (2.8%), with a 39.3% survival rate and a high rate of nonrepairs (50.1%). The incidence of birth at <34 weeks of gestational age for those undergoing fetal tracheal occlusion was 30%.

Conclusions

Survival rates for infants born preterm with CDH remain low and the implementation of fetal tracheal occlusion is likely to increase the rate of prematurity in CDH. The high rate of nonrepair is a significant contributor to lower survival, and the more aggressive use of extracorporeal life support and surgical repair may improve survival.